Classic divided sigmoidostomy vs loop sigmoidostomy in anorectal malformations: time for change?

PURPOSE: Divided sigmoidostomy (DS) is the classic stoma for patients with anorectal malformations (ARM). Loop sigmoidostomies (LS) in ARM are associated with a higher risk of stoma prolapse and urinary tract infections (UTI). This is not clearly supported by literature. We compared our experience with both techniques. METHODS: Retrospective study of ARM patients who underwent DS or LS between 2013 and 2023. We analysed demographics, associated malformations, intraoperative variables, oral intake and stoma functioning times, hospital stay, complications, prolapses, and UTI. RESULTS: Of 40 patients, 29 underwent open DS and 11 laparoscopic LS. Demographics, malformation type, associated anomalies, surgical time, intraoperative and anaesthetic complications were comparable. Postoperative complications were higher in DS than LS [14(48.3%) vs 1(9.1%), (p = 0.02)], mostly due to wound complications [12(41.3%) vs 0(0%), (p = 0.01)]; with 3 dehiscenses and 3 strictures reintervened. The hours to oral intake and stoma functioning were higher for DS [48(39-90) and 48(24-48) vs 24(24-48) and 24(24-24), (p < 0.05)], with more days of hospital stay [36(19-60) vs 8(5-10), (p = 0.001)]. Prolapses [1(3.4%) vs 1(9.1%)] and UTIs [3(10.3%) vs 1(9.1%) (p > 0.05)] were comparable. CONCLUSION: LS in ARM patients have no higher risk of prolapse or UTI than DS. DS had more complications, mostly wound infections, strictures and dehiscenses.

[Treatment of rectal prolapse after proctoplasty in a child with rectal atresia]. / Lechenie vypadeniya pryamoi kishki posle proktoplastiki u rebenka s atreziei pryamoi kishki.

Rectal prolapse is a common disease in childhood and observed mainly at the age of 1-4 years old (95% of cases). If conservative treatment is ineffective, surgical correction of rectal prolapse in children without previous anorectal surgery is performed at the age of over a year. There is a single report on examination of patients aged 4-16 years after surgical correction of anorectal malformations with postoperative rectal prolapse. We present diagnosis and successful surgical treatment of rectal prolapse in an infant who underwent previous perineal proctoplasty for fistulous form of anorectal malformation.

Female Anorectal Malformation with Genitourinary Prolapse: A Rare Association.

ABSTRACT: Genitourinary prolapse in newborn females as an introital mass is an uncommon entity. The usual causative mechanisms are poor pelvic innervation, damage or pressure on pelvic musculature and ligaments etc. Different methods of reduction as treatment were proposed in the past. Apart from uncommon occurrence of genitourinary prolapse in newborns, its association with anorectal malformation is not reported in English literature after searching on PubMed and Google Scholar. We report three cases of genitourinary prolapse with anorectal malformation in newborn females where decompressing colostomy was curative for the condition reflecting increased intra-abdominal pressure as causative mechanism.

Current practice of diagnosis and treatment for rectourethral fistula in male patients with anorectal malformation: a multicenter questionnaire survey in Japan.

PURPOSE: Surgical procedures for anorectoplasty for anorectal malformations (ARMs), particularly rectourethral fistula (RUF), depend on the institution. We investigated the diagnosis and treatment of RUF in male patients with ARMs in Japan using a questionnaire survey. METHODS: An online survey inquiring about the diagnosis and treatment (diagnostic modalities, surgical approaches, fistula dissection devices, and fistula closure techniques) of each type of ARM in male patients was conducted among institutional members of the Japanese Study Group of Anorectal Anomalies. Fisher's exact test was used to compare surgical methods between posterior sagittal anorectoplasty (PSARP) and laparoscopy-assisted anorectoplasty (LAARP). RESULTS: Sixty-one institutions (100%) completed the survey. LAARP was the preferred approach for high-type ARM (75.4%). PSARP was preferred for intermediate-type ARM (59.0%). Monopolar devices were most commonly used (72.1%) for RUF dissection. Blunt dissection was more frequent in the PSARP group (PSARP vs. LAARP: 55.6 vs. 20.0%, p < 0.005). Cystoscopy/urethroscopy to confirm the extent of dissection was used more frequently in the LAARP group (70.0% vs. 25.0%, p < 0.005). Clips and staplers were used more frequently in the LAARP group (p < 0.05). CONCLUSION: Distinct fistula management strategies for PSARP and LAARP were revealed. Further studies are needed to investigate the postoperative outcomes associated with these practices.

Commentary- Bridging the Gap: The Impact of Bowel Management Programs on Daily Life in Pediatric Colorectal Disease.

BACKGROUND: This commentary discusses the social impact of bowel management programs (BMPs) on children with colorectal diseases, including anorectal malformations (ARM), Hirschsprung disease (HD), functional constipation (FC), and spina bifida. Previous studies focused on functional outcomes, but this study bridges the gap to daily life experiences. METHODS: The study examined children's experiences in BMPs, focusing on school participation, vacation ability, and overall patient experience. Cleanliness, defined as fewer than one stool soiling episode per week, was achieved by 70% of participants. RESULTS: Positive patient experiences were linked to achieving stool cleanliness, regardless of the management method. Invasive methods like enemas did not negatively affect experiences if cleanliness was maintained. Validated patient-reported experience measures (PREMs) and patient-reported outcomes measures (PROMs) were used, though the median age of 8.9 years posed limitations. CONCLUSION: The commentary highlights the significance of stool cleanliness in improving patient experiences and supports the effectiveness of various BMP methods. Future research should include longitudinal follow-ups to assess BMP durability and gather data from older children.

Early post-operative fever after colorectal surgery in infants is common and rarely associated with infection.

PURPOSE: Early post-operative fever (< 48 h) is common in adults and children and seldom indicative of an infection. Guidance to limit excessive evaluation in adults is well-characterized but similar studies for the pediatric population is scarce. This study was performed to better clarify which infants should undergo investigation for post-operative fever after colorectal reconstructive surgical procedures. METHODS: We performed a retrospective chart review of all infants under one year of age who underwent elective reconstruction for anorectal malformations (ARM) and Hirschsprung Disease (HD) between June 2018 and April 2020 at a single institution. Patient and perioperative characteristics were analyzed to evaluate for possible factors associated with infection. RESULTS: Sixty-eight infants met study criteria - 38 (55.9%) had HD and 30 (44.1%) had ARM. Twenty-two infants (32.4%) had early post-operative fever. A definitive infectious cause was identified in only two infants. The presence of a colostomy pre-operatively and longer operative times were associated with increased risk of post-operative fever (62.5% vs. 22.7% and 175 min vs. 150 min respectively, p < 0.05). CONCLUSION: Early post-operative fever in infants after colorectal surgery is common and rarely associated with an infection. Further research is needed to determine which infants require further work-up and which can be safely observed.

Classification and Surgical Management of Anorectal Malformations: A Systematic Review and Evidence-based Guideline From the APSA Outcomes and Evidence-based Practice Committee.

OBJECTIVE: Treatment of neonates with anorectal malformations (ARMs) can be challenging due to variability in anatomic definitions, multiple approaches to surgical management, and heterogeneity of reported outcomes. The purpose of this systematic review is to summarize existing evidence, identify treatment controversies, and provide guidelines for perioperative care. METHODS: The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee (OEBP) drafted five consensus-based questions regarding management of children with ARMs. These questions were related to categorization of ARMs and optimal methods and timing of surgical management. A comprehensive search strategy was performed, and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to perform the systematic review to attempt to answer five questions related to surgical care of ARM. RESULTS: A total of 10,843 publications were reviewed, of which 90 were included in final recommendations, and some publications addressed more than one question (question: 1 n = 6, 2 n = 63, n = 15, 4 n = 44). Studies contained largely heterogenous groups of ARMs, making direct comparison for each subtype challenging and therefore, no specific recommendation for optimal surgical approach based on outcomes can be made. Both loop and divided colostomy may be acceptable methods of fecal diversion for patients with a diagnosis of anorectal malformation, however, loop colostomies have higher rates of prolapse in the literature reviewed. In terms of timing of repair, there did not appear to be significant differences in outcomes between early and late repair groups. Clear and uniform definitions are needed in order to ensure similar populations of patients are compared moving forward. Recommendations are provided based primarily on A-D levels of evidence. CONCLUSIONS: Evidence-based best practices for ARMs are lacking for many aspects of care. Multi-institutional registries have made progress to address some of these gaps. Further prospective and comparative studies are needed to improve care and provide consensus guidelines for this complex patient population.

Management of urinary incontinence in girls with congenital pouch colon.

PURPOSE: This study describes the management of urinary incontinence (UI) in eight girls with congenital pouch colon (CPC) associated with anorectal malformation (ARM). METHODS: From 2013 to 2015, six girls with CPC and UI underwent bladder neck reconstruction (BNR). Four girls had complete UI (CUI) and two girls partial UI (PUI). From 2019 to 2023, four girls, including two with failed BNR, underwent bladder neck closure (BNC) and augmentation cystoplasty (AC) with a continent stoma. Subtypes of CPC were Complete CPC (n = 7) and Incomplete CPC (n = 1). All girls had a double vagina; short, wide urethra; and reduced bladder capacity with an open, incompetent bladder neck (BNI). During BNR, a neourethra was constructed from a 1.5-2 cm-wide and 1.5-3-cm-long trigonal strip. During BNC, AC was performed using a 20 cm ileal segment (n = 3) and by a colonic pouch segment, preserved during earlier colorraphy (n = 1). Continent stoma included a Monti's channel (n = 3) and appendicovesicostomy (n = 1). RESULTS: BNR produced moderate improvement of UI (n = 2), while UI was still very severe (n = 4). During BNC, intraoperative complications included iatrogenic vaginal tears (n = 4). Early complications included partial dehiscence of the ileocystoplasty (n = 1), partial adhesive small bowel obstruction (n = 1), and difficulty in stomal catheterization with prolonged drainage from the pelvic drain (n = 1). Late complications included unilateral grade II vesicoureteric reflux (n = 2) and vesicovaginal fistula (VVF) (n = 2) needing trans-vaginal closure in one girl. Urinary stones (n = 2) with stomal leakage of urine in one girl needed open cystolithotomy twice (n = 1), and endoscopic lithotripsy (n = 1). At follow-up, all patients have high overall satisfaction with the procedure and their continence status. CONCLUSIONS: BNC with AC and a catheterizable stoma satisfactorily achieves continence in girls with CPC and UI, vastly improving quality of life. If lower urinary tract (LUT) anatomy is favorable, BNR with/without AC can be the initial surgical procedure. BNC should be the primary procedure in girls with unfavorable LUT anatomy and for failed BNR. LEVEL OF EVIDENCE: IV.

Late diagnosis of anorectal malformation: how good is good enough?

PURPOSE: National data from the United Kingdom reported in 2016 have suggested that almost one quarter of babies with anorectal malformation (ARM) have a delay in diagnosis. The UK's Newborn Infant Physical Examination dictates a perineal examination should be performed within 72 h of birth. We sought to describe a tertiary single-centre experience of late presentation in the most recent 5 years. METHODS: A single-centre prospective registry of ARM patients (July 2018-March 2024) was analysed. Timing of presentation with anomaly was noted. Patients presenting > 72 h or having been discharged home were defined as a delayed diagnosis. Factors associated with delayed diagnosis were noted. RESULTS: Sixty patients were included, of whom nine (15%) were diagnosed after 72 h [range 4-279 days]. This represents a non-significant improvement compared to 39/174 (22%) late diagnosed cases in the BAPS-CASS cohort from 2016 to 17 (p = 0.188). Presenting symptoms of obstruction (i.e. distension, vomiting, megarectum) were more common in late diagnosed patients (4/9 (44%) vs. 1/51(2%); p = 0.001). Anomalies producing meconium on the perineum were more likely to be diagnosed late (8/32 (25%) vs 1/28 (4%); p = 0.029). Complications and changes to clinical management for these cases are presented. CONCLUSION: Although our regional rates of late diagnosis appear to be lower than previously reported national rates, there remains a significant number of infants who are diagnosed late especially those with visible perineal openings. These infants are more commonly symptomatic; entraining additional risks associated with an emergency presentation.

Robotic-assisted pull-through procedures for anorectal malformation: a systematic review.

AIM: To study the published literature on robotic-assisted pull-through procedures for anorectal malformation. METHOD: A PubMed search was done on 10th April 2024, with the words "robotic AND Anorectal malformation". The articles were screened for relevance and the data were compiled on the safety, feasibility, technical details, and limitations of robotic-assisted procedures in children for anorectal malformation. RESULTS: The search robotic and anorectal malformation gave ten articles. Two were excluded as they were not relevant. Two articles were added from cross-reference. Ten publications on robotic-assisted procedures for anorectal malformation were studied, describing procedures in thirty-three cases. The youngest child operated was 3 months old. All except one case were done in males. Most articles were from the US and the Kingdom of Saudi Arabia (KSA). The principles involved in robotic-assisted anorectoplasty (RAARP) were the same as that of laparoscopic procedures. Complications reported included pelvic abscess, epididymo-orchitis, and stricture of pelvic tunnel or conversion to open. The magnification and endo-wrist technology of robotics facilitated the sharp dissection and ligation at origin of fistula. The mean operating time was 228.7 min (docking and console time), shortest being 86 min and mean hospital stay was 7 days. The number of ports used varied from 3 to 4 with 8.5 mm being the most commonly used umbilical port and 8 mm as working ports, although in one article, a 12 mm port was used for telescope. The prolonged operating time and cost are the two factors to be addressed in RAARP. CONCLUSION: Robotic surgery is feasible in infants with ARM and safe in expert hands. Robotics is a very effective tool with its better ergonomics, tremor filtration, 3D magnification, and dexterity. Increasing awareness and referral to high-volume centers can tide over the cost factor, and good training of the surgeons can reduce the operative time.

Simulation and training for pediatric colorectal surgery and anorectal malformation: a scoping review.

PURPOSE: To study the published literature for various models used for simulation and training in the field of pediatric colorectal surgery. METHOD: A PubMed search was conducted for studies of simulation models in anorectal malformation on 24 March 2024 with the search words 'simulation pediatric colorectal surgery' followed by another search on 'simulation AND anorectal malformation' that gave 22 and 14 results, respectively (total 36). After removing 4 duplicate publications, 12 were found relevant to simulation and training in colorectal diseases. One publication relevant to the topic was added from literature, thirteen articles were studied. RESULTS: Of these, 5; 1; 4; and 3 were on inanimate models; animate model; 3D reconstructions; and training, respectively. Simulation models are available for posterior sagittal anorectoplasty. The same inanimate model was used in five articles. The animate model was based on a chicken cadaver. 3D models have been made for personalized preoperative assessment and to understand the imaging in anorectal malformation. One 3D model was made by regeneration of organoid epithelium. Training modules were made to evaluate surgical dissection, standardize surgical techniques, and improve proficiency. CONCLUSION: Simulation models are an important tool for teaching the steps of surgery and discussing the nuances of operative complications among mentors and peers. With advances in this field, the development of high-fidelity models, more training modules, and consensus on surgical techniques will benefit surgical training.

A multi-center cross-sectional comparison of parent-reported quality of life and bowel function between anorectal malformation and Hirschsprung's disease patients with versus those without Down syndrome.

PURPOSE: Down syndrome (DS) is a common abnormality associated with anorectal malformation (ARM) and Hirschsprung's disease (HD). However, quality of life (QOL) in ARM and HD patients with DS is under-researched. This study compares parent-reported QOL and bowel function in ARM and HD patients with DS to those without. METHODS: Between December 2020 to February 2023, parents of ARM and HD patients with and without DS aged 3-17 years who had undergone surgery > 12 months prior at four tertiary referral centers were recruited. We used the Pediatric Quality of Life Inventory™ (PedsQL™) Generic Core Scales, General Well-Being (GWB) Scale and Family Impact (FI) Module questionnaires, and the Rintala bowel function score (BFS). RESULTS: There were 101 ARM, 9 (8.9%) of whom had DS; and 87 HD, of whom 6 (6.9%) had DS. Parent-reported Core scores in ARM and HD with DS were comparable to those without DS. However, ARM and HD with DS had worse scores in the FI Module and bowel function than those without DS. CONCLUSION: Although parent-reported QOL in ARM and HD with DS is similar to those without DS, family impact and BFS are worse. Our findings are limited by small sample size in proportion of DS patients.

Assessing fecal load with ultrasound in children with colorectal pathology: ReKiSo study.

PURPOSE: To evaluate bowel management for children with colorectal pathology by measuring transverse rectal diameter (TRD) and assessing fecal load with transabdominal rectal ultrasound (TRU). METHODS: Prospective case-control study of children receiving bowel management (BM) between 04/2023 and 04/2024 was done. There was inclusion of patients with Hirschsprung disease (HD), anorectal malformation (ARM) and functional constipation (FC). Patients with other congenital or neurological conditions were excluded. Control group consisted of inpatients and outpatients without abdominal complaints. FC was diagnosed according to ROM-IV-criteria. For HD and ARM, we followed a list of symptoms. To assess fecal load, we visualized the TRD using the Klijn (Klijn et al. in J Urol 172:1986-1988, 2004) method. The bladder was moderately full. The fecal load was assessed retrograde from the rectum. Follow-up was at 1/3/6 months. Secondary data were collected from medical records. Sample size calculated a priori and follow-up group with new gathered data. RESULTS: p value for TRD in all groups significant with p < 0.05 and in grouped follow-up. CONCLUSION: Ultrasound is a useful tool for assessing fecal load and helps diagnose constipation and monitor BM. Irrespective of colorectal pathology, a cut-off of 3 cm seems to discriminate between children without constipation/overload symptoms and asymptomatic patients. We present a radiation-free method for monitoring bowel management.

Urological outcomes in adult females born with anorectal malformation or Hirschsprung disease.

INTRODUCTION: Women born with anorectal malformation (ARM) or Hirschsprung disease (HD) may have impaired urologic function resulting in sequelae in adulthood. This study assessed and compared self-reported urinary outcomes in adult females born with ARM or HD to a reference population. METHODS: This was an IRB approved, cross-sectional study of female-born patients with ARM or HD, who completed surveys between November 2021 and August 2022. Female patients between the ages of 18 and 80 years were included. Lower Urinary Tract Symptom Questionnaires were administered through REDCap and the responses were compared to a reference population using Chi-squared or Fisher's exact tests. RESULTS: Sixty-six born female patients answered the questionnaires, two of them identified as non-binary. The response rate was 76%. Median age was 31.6 years. The majority were born with cloaca (56.3%), followed by other type of ARMs (28.1%), complex malformation (9.4%), and HD (6.3%). A history of bladder reconstruction was present for 26.6%. Catheterization through a channel or native urethra was present in 18.8%. Two had ureterostomies and were excluded from the analysis. Seven had chronic kidney disease or end-stage renal disease, three with a history of kidney transplantation. Patients with cloaca had significantly higher rates of urinary incontinence, urinary tract infection, and social problems due to impaired urological functioning, when compared to an age-matched reference population (Table 3). CONCLUSION: This study emphasizes the need for a multi-disciplinary team that includes urology and nephrology following patients with ARM long term, especially within the subgroup of cloaca. LEVEL OF EVIDENCE: III.

A novel SALL1 C757T mutation in a Chinese family causes a rare disease --Townes-Brocks syndrome.

BACKGROUND: Townes-Brocks syndrome (TBS) is a rare genetic disorder characterized by imperforate anus, dysplastic ears, thumb malformations, and other abnormalities. Previous studies have revealed that mutations in the SALL1 gene can disrupt normal development, resulting in the characteristic features of Townes-Brocks syndrome. Spalt-like transcription factors (SALLs) are highly conserved proteins that play important roles in various cellular processes, including embryonic development, cell differentiation, and cell survival. Over 400 different variants or mutations have been reported in the SALL1 gene in individuals with TBS. Most of these variants lead to the formation of premature termination codons (PTCs), also known as nonsense mutations. The majority of these PTCs occur in a specific region of the SALL1 gene called the "hotspot region", which is particularly susceptible to mutation. METHODS: In this study, we conducted whole-exome sequencing on a three-generation Chinese family with anorectal malformations. RESULTS: We identified a novel heterozygous mutation (chr16:51175376:c.757 C > T p.Gln253*) in the SALL1 gene. Molecular analysis revealed a heterozygous C to T transition at nucleotide position 757 in exon 2 of the SALL1 (NM_002968) gene. This mutation is predicted to result in the substitution of the Gln253 codon with a premature stop codon (p.Gln253*). The glutamine-rich domain forms a long alpha helix, enabling the mutant protein to interact with the wild-type SALL1 protein. This interaction may result in steric hindrance effects on the wild-type SALL1 protein. CONCLUSIONS: Our findings have expanded the mutation database of the SALL1 gene, which is significant for genetic counseling and clinical surveillance in the affected family. Furthermore, our study enhances the understanding of Townes-Brocks syndrome and has the potential to improve its diagnosis and treatment.

A Child With Diphallia, Duplicate Bladder, Bladder Exstrophy, and Anorectal Malformation.

Congenital true diphallia, complete duplicate bladder, bladder exstrophy, and anorectal malformation in a child are uncommon. Here, we present the case of a 3-year-old boy with multiple genitourinary malformation, including true diphallia, complete duplicate bladder, bladder exstrophy, epispadias, and anorectal malformation. Multi-departmental collaborative treatment for complex conditions ultimately achieved an ideal appearance for this patient. All vital signs were stable after the surgery and they remained consistent during follow-up. In such cases, surgical correction is individualized to achieve adequate urinary continence and erection with adequate esthetics.

Genital Malformations in Children With VACTERL - Has Time Come to Include "G" in the Acronym?

BACKGROUND: Genital malformations are frequently diagnosed in patients with VACTERL, but are currently not included in the acronym. This study aimed to analyze the frequency of genital anomalies in patients with esophageal atresia (EA) and/or anorectal malformation (ARM), with a subgroup analysis of children fulfilling the VACTERL criteria. METHOD: This was a cross-sectional retrospective analysis of two prospectively collected registries of patients operated on for ARM and EA between 2012 and 2022 at a specialized national center. Children were screened routinely for malformations according to the VACTERL acronym. RESULTS: A total of 174 children were included in the study. VACTERL was diagnosed in 60 children (34%), while 114 children (66%) were defined as non-VACTERL. Genital malformations were diagnosed in 38% (23/60) of the children with VACTERL, and in 11% (13/114) of the children without VACTERL (p < 0.001). The presence of genital malformations correlated linearly with the number of diagnosed component features (CFs). In boys with VACTERL, the most common genital malformation was undescended testes present in 10/27 (21%) compared to 1/71 (1%) in non-VACTERL boys (p < 0.001). Müllerian duct anomalies were found in 26% of girls with VACTERL vs. 7% in non-VACTERL girls (p < 0.05). CONCLUSION: There was a higher frequency of genital malformations in patients with VACTERL emphasizing the importance of genital assessment for these patients. We propose VACTERL-G as an extension of the current acronym aiming to reduce the risk of long-term morbidity due to delayed diagnosis of reproductive anomalies.

Neighborhood-level Disparities in Achievement of Social Continence Among School-aged Children With Anorectal Malformations: A Single-center Retrospective Study.

BACKGROUND: Fecal incontinence is a common problem for children with repaired anorectal malformations (ARM) and has significant implications for initiating school. While sex, anatomy, and medical comorbidities are known to influence continence outcomes, the impact of socioeconomic factors and neighborhood-level disadvantage are less well understood. METHODS: We performed a single-center retrospective review of all school-aged (5-18 years) children with ARM at a longitudinal pediatric surgery clinic. Demographic, clinical, and socioeconomic variables were abstracted via chart review and geocoding was performed to obtain Area Deprivation Index (ADI) and Social Vulnerability Index (SVI) scores. Statistical analyses assessed for associations between the primary outcome of social continence (defined as no diaper usage and infrequent fecal accidents at age 5) and these variables. RESULTS: 72 patients were included; of these, 45.8% were socially continent. On bivariate analysis, social continence was significantly associated with state ADI score as well as the SVI Housing characteristics score. These associations remained significant when adjusting for sex and medical comorbidities in separate multiple logistic regression models. CONCLUSION: The relative disadvantage of the neighborhood in which a child with ARM lives may play a role in their ability to achieve continence by school age. Efforts are warranted to identify and develop targeted interventions to for this pediatric population. LEVEL OF EVIDENCE: IV.

Bowel function, urinary tract function, and health-related quality of life in males with anorectal malformations.

PURPOSE: There is a knowledge gap regarding long-term outcomes for males undergoing surgery for an anorectal malformation (ARM). The purpose of this study was to investigate bowel function, bladder function, and health-related quality of life (HRQoL) in male patients with an anorectal malformation. METHODS: This cross-sectional questionnaire-based study included males treated for ARM at our institution between 1994 and 2017. Bowel function was assessed with bowel function score (BFS) while urinary tract function was assessed with lower urinary tract symptoms (LUTS) questionnaires. Health-related quality of life (HRQoL) was investigated using age-relevant questionnaires (KIDSCREEN and PGWBI). Patient characteristics were retrospectively collected from the medical records and descriptive statistics were used for analysis. Functional outcomes were compared with gender and age-matched controls while HRQoL was compared to normative data. The study was approved by ethics review authorities. RESULTS: A total of 58 (44.6%) of 130 males responded to the questionnaires. Regarding bowel function, 24 (42.1%) of 57 patients and 81 (95.3%) of 85 controls, respectively, reported a well-preserved bowel function represented by a BFS ≥ 17 (p < 0.001). Soiling issues and 'feels urge' items improved significantly with age. In a linear regression model, BFS increased significantly with age. For most parameters, the proportion of ARM patients with lower urinary tract symptoms was larger, though not significantly, compared to the controls. However, straining and stress incontinence were reported significantly more often by ARM patients. In patients and controls, voiding outcomes in terms of prevalence of having symptoms and the number of cumulative symptoms drop with increasing age. Children and adults reported similar or, in some domains, better HRQoL outcomes when compared to normative European data. CONCLUSION: Bowel function is impaired in male patients with ARM but significantly improves with age. Urinary tract function was affected, but overall comparable to the controls. HRQoL was unaffected. No significant association between the studied outcomes could be shown. LEVEL OF EVIDENCE: III.